Isolated Fracture of the Malleus: An Overlooked Cause of Conductive Hearing Loss?

OBJECTIVE: To report the largest case series of isolated malleus fractures with systematic review to characterize the disease's presentation and natural history, and provide suggestions for management. DATA SOURCES: PubMed, Embase, Cochrane Library. REVIEW METHODS: Retrospective cohort study was performed on 12 patients with isolated malleus fractures. History, physical exam, pre- and post-treatment audiograms, and imaging were obtained. Systematic review of the literature was performed. RESULTS: Including the cases herein, 58 isolated malleus fractures were identified, the majority of which were published in the 21st century. Mean time to presentation after injury was 34.4 months. Most common etiology was external auditory canal (EAC) manipulation. Physical exam and imaging did not identify any abnormality at presentation in 16% and 21% of cases, respectively. The majority of fractures involved the manubrium. Air-bone gap (ABG) at initial presentation ranged from 16 to 26 dB, and was greater at higher frequencies. Thirty-six cases underwent surgery. ABG improvement was greater at all frequencies for those who underwent surgery. Final ABG was significantly less than initial ABG at nearly every frequency for those who underwent surgery (p < 0.05), while not at any frequency for those who were observed. CONCLUSIONS: Isolated malleus fractures may occur more often than historical data suggests, and are perhaps underdiagnosed. Abrupt removal of a finger from the EAC with pain and hearing loss is nearly pathognomonic. Conductive hearing loss with ABG greater at higher frequencies is most often observed. Observation is unlikely to produce spontaneous improvements in hearing, while surgery demonstrates reliable decreases in ABG. Laryngoscope, 134:1032-1041, 2024.

Spontaneous Congenital Perilabyrinthine Cerebrospinal Fluid Fistulas.

OBJECTIVES: To report a recalcitrant spontaneous cerebrospinal fluid (CSF) fistula arising from multiple, anatomically-linked lateral skull base defects, and to review the available literature to determine optimal techniques for operative repair of congenital CSF fistulae. METHODS: A patient with recurrent episodes of otologic meningitis was found to have a patent tympanomeningeal fissure, also known as a Hyrtl's fissure, and internal auditory canal (IAC) diverticulum that communicated with the jugular bulb. A systematic review of the literature characterized all reports of spontaneous congenital perilabyrinthine CSF leaks, and all cases of Hyrtl's fissures. RESULTS: An 11-year-old female was referred for recurrent meningitis. Imaging demonstrated a fistulous connection between the middle ear and IAC diverticulum via the jugular foramen. Specifically, a Hyrtl's fissure was identified, as well as demineralized bone around the jugular bulb. Obliteration of the fissure was initially performed, and a fistula reformed 4 months later. Multifocal CSF egress in the hypotympanum was identified on re-exploration, and middle ear obliteration with external auditory canal (EAC) overclosure was performed. A systematic review of the literature demonstrated 19 cases of spontaneous congenital perilabyrinthine CSF leaks. In total, 6 cases had multiple sources of CSF leak and 2 had history suggestive of intracranial hypertension. All of these noted cases demonstrated leak recurrence. Middle ear obliteration with EAC overclosure was successful in 4 recalcitrant cases. CONCLUSIONS: Repair of spontaneous congenital perilabyrinthine CSF leaks in cases demonstrating multiple sources of egress or signs of intracranial hypertension should be approached with caution. Middle ear obliteration with EAC overclosure may provide the most definitive management option for these patients, particularly if initial attempt at primary repair is unsuccessful.

Melanoma of the External Auditory Canal: A Review of Seven Cases at a Tertiary Care Referral Center.

OBJECTIVES/HYPOTHESIS: Examine the presentation and management characteristics of seven patients with melanoma of the external auditory canal (EAC). STUDY DESIGN: Retrospective case series and review of the relevant literature. METHODS: Records of seven patients from 2003 to 2017 with melanoma of the EAC were reviewed for characteristics of presentation, subsequent management, and outcomes. A thorough review of relevant literature is presented. RESULTS: The median age is 52 years, with four females. The average Breslow depth was 3.6 mm, with five patients having a Clark level IV or greater on presentation. Six patients underwent lateral temporal bone resection, and one patient underwent wide local excision of the cartilaginous canal. Sentinel lymph node biopsy (SLNB) was performed in three patients. Three patients experienced distant recurrence an average of 20 months following primary therapy. Median follow-up was 21 months. At last follow-up, four were free of disease, one had active disease, and two were deceased from melanoma. CONCLUSIONS: This is the largest series and the first to report the use of SLNB for patients with EAC melanoma in the peer-reviewed literature. Patients with external auditory canal melanoma present with higher Breslow thickness and stage relative to all external ear melanomas. Management should include wide local excision, which entails lateral temporal bone resection when the bony ear canal is involved. SLNB has a critical role in identifying patients with early metastatic disease. Postoperative radiation therapy should be considered for patients with high-risk features to reduce the risk of locoregional relapse. Chemotherapy, and especially immunotherapy, has an emerging role for this disease. LEVEL OF EVIDENCE: 4 Laryngoscope, 131:165-172, 2021.

Otosclerosis in an Urban Population.

OBJECTIVE: To investigate the evolving prevalence of otosclerosis in a large urban population. METHODS: A retrospective review of patients in a large, urban, public health system was conducted from January 2010 to August 2019 to identify subjects with otosclerosis. Diagnostic testing included audiometry and computed tomography scans. Sex, age at diagnosis, treatment received, race, ethnicity, and country of birth were analyzed for each subject and compared with all eligible patients in the reference population. RESULTS: A total of 134 patients from a reference population of 672,839 were diagnosed with otosclerosis and analyzed. The otosclerosis patients were predominantly Hispanic (73%), of which the majority were foreign born (87%). The average age at onset was 46 years and 59% were women. The overall prevalence of otosclerosis was 20 of 100,000 patients. The crude prevalence of otosclerosis by ethnicity was 43 of 100,000 for Hispanics, 12.6 of 100,000 for Caucasians, and 3 of 100,000 for African Americans. Within the Hispanic population, the prevalence of otosclerosis was 60 of 100,000 for foreign-born individuals and 16 of 100,000 for those born in the USA (odds ratio [OR] = 3.69, [95% confidence interval [CI], 2.02-6.76], p < 0.0001). Prevalence was not significantly different among Caucasians and US-born Hispanics. CONCLUSION: Otosclerosis in the studied population was most common among Hispanic patients, though it was strongly influenced by country of birth. The "imported" otosclerosis cases are best explained by environmental influence rather than ethnic susceptibility. This discrepancy is likely due to variance in measles immunization rates among North and Central American countries before 1990.

A Systematic Review of Cochlear Implant Outcomes in Prelingually-deafened, Late-implanted Patients.

OBJECTIVE: To analyze outcomes of cochlear implantation (CI) in prelingually-deafened, late-implanted patients. DATA SOURCES: A search of MEDLINE and EMBASE was performed in February 2018 using SCOPUS for the intersection of "cochlear implant," "prelingual," "deaf," and "delay." REVIEW METHODS: Two independent reviewers screened all abstracts and titles for relevance, with conflicts resolved by either the primary or senior author. All articles passing this screen were subjected to a full-text review, during which the primary and senior author each examined manuscripts for inclusion and exclusion criteria. The Cochrane Risk of Bias Assessment Tool was used to assess potential sources of systematic error, and postoperative clinical outcomes were collected at the latest clinical follow-up. RESULTS: Twenty-eight articles were yielded in the final systematic review, accounting for 542 patients. For open-set sentence scores, 10 studies representing 240 patients showed an overall estimated improvement of 44.6% (95% CI: 38.0-51.2%). In terms of quality of life, studies generally showed improvement when looking at specific emotional, social, or hearing-specific domains, but not in global measures. Nonuser rates ranged from 0 to 9.5%. CONCLUSION: Despite performance that is generally poorer than what is generally seen in "traditional" candidates, prelingually-deafened, late-implanted (PL-LI) CI users can experience benefit in terms of both QOL and audiometric scores. The wide range of performance that is reported in the literature highlights the importance of careful patient counseling.

Microvascular free tissue transfer and cochlear implants: A case series and literature review.

OBJECTIVE: The use of microvascular free tissue transfer (MVFTT) for defects of the scalp and lateral temporal bone in patients with cochlear implants (CI) is uncommon. Herein, we report our experience with the utility, indications, and outcomes for MVFTT in patients with cochlear implants. METHODS: A retrospective review of patients at our institution from September 2016 to December 2017 identified subjects with coexistent cochlear implant and ipsilateral MVFTT of the lateral temporal bone or scalp. Information including demographics, indication for MVFTT, timing of CI and MVFTT, donor site, and previous radiation to the head and neck was collected. To assess the current literature on MVFTT in CI patients, a MEDLINE search was performed using key search terms. RESULTS: Two patients with cochlear implants and MVFTT of the ipsilateral temporal bone or scalp were identified. One patient underwent MVFTT for advanced device extrusion with stable audiometric parameters rather than locoregional reconstruction or device explantation. The second patient had primary cochlear implantation at oncologic lateral temporal bone resection (LTBR) and MVFTT for locally advanced squamous cell carcinoma and concurrent profound sensorineural hearing loss (SNHL). A literature review identifies MVFTT as an option for advanced device extrusion, treatment of osteoradionecrosis, and reconstruction after primary oncologic surgery. CONCLUSION: MVFTT is an important reconstructive tool for patients with functional, exposed cochlear implants. Cochlear implantation for severe to profound SNHL should be considered at the time of primary oncologic surgery and MVFTT of the lateral temporal bone or scalp. LEVEL OF EVIDENCE: IV Laryngoscope, 130:1552-1557, 2020.

The Role of Obesity in Iatrogenic Encephaloceles of the Temporal Bone.

OBJECTIVE: Acquired encephaloceles of the temporal bone may be traumatic, spontaneous, or from chronic ear disease or previous surgery. Iatrogenic encephaloceles arise in the setting of previous mastoidectomy and traditionally involve both bony dehiscence and dural injury. We aim to classify and analyze the pathogenesis, clinical presentation, and treatment options for patients with iatrogenic tegmen defects and encephaloceles of the temporal bone. STUDY DESIGN: Retrospective chart review. SETTING: Single tertiary academic center. PATIENTS: Subjects with iatrogenic tegmen defects and encephaloceles of the temporal bone were included. INTERVENTION: Patient demographics, history, symptoms, radiographic data, intraoperative findings, management, follow-up, and outcomes were recorded. OUTCOME MEASURES: Primary outcome measures included patient characteristics, time from primary otologic surgery to surgical repair, location of the defect, and management strategy including surgical approach, methods, and follow-up. RESULTS: Iatrogenic tegmen injuries or encephaloceles were identified in 18 patients and divided into intentional or unintentional. The latter group presented immediately, early, or late, as determined by intraoperative identification or from delayed symptoms. Eleven patients presented late with previously unrecognized encephaloceles. Compared with patients presenting with incidentally noted tegmen dehiscence during revision mastoidectomy, a significant proportion of patients with late encephaloceles had BMI ≥30 kg/m (p = 0.03). CONCLUSION: The majority of iatrogenic encephaloceles are unintentional, unrecognized, and may present many years after primary surgery. Similar to spontaneous encephaloceles of the temporal bone, obesity is associated with iatrogenic encephaloceles, although the rate of progression and timing is unclear. We recommend primary repair of any unintentional tegmen defect encountered during mastoid surgery, especially in obese patients.

Postoperative MRI Surveillance of Vestibular Schwannomas: Is There a Standard of Care?

OBJECTIVE: To examine current practices for postoperative imaging surveillance following vestibular schwannoma resection. STUDY DESIGN: Cross-sectional survey of practicing neurotologists. SETTING: Tertiary referral centers. PATIENTS: Not applicable. INTERVENTION: Two hundred seventy-six members of the American Neurotology Society were invited to participate. Using a web-based format, respondents self-reported demographic and practice details. Case scenarios were presented. For each scenario, both quantitative and qualitative data were recorded. MAIN OUTCOME MEASURES: Timing, frequency, duration, and modality of postoperative imaging. RESULTS: For all scenarios, responses were widely disparate with respect to timing, frequency, number, and duration of follow-up imaging. Following gross total resection, respondents most commonly (46.5%) obtain the first magnetic resonance imaging 1 year after surgery, with the most common endpoint to discontinue surveillance imaging at 10 years (34.9%). Tumor beds with postoperative enhancement were generally followed longer than those without enhancement, but with wide variability in practice patterns. The majority of neurotologists do not change surveillance patterns regarding tumor size or completeness of initial resection. Lower volume surgeons appear to be more aggressive with initial surveillance postoperatively than higher volume surgeons. CONCLUSION: Wide variability exists amongst neurotologists concerning postsurgical tumor surveillance imaging. Despite recent data to suggest more standardized protocols, disparate practice patterns persist. Additional research is needed to this end, as the subsequent establishment of such evidence-based protocols could obviate substantial medical, medico-legal, and economic burdens concerning this patient population.

Manifestations of Skull Base IgG4-Related Disease: A Multi-Institutional Study.

OBJECTIVE: IgG4-related disease (IgG4-RD) is a recently recognized disease characterized by fibroinflammatory infiltrates rich in IgG4+ plasma cells that can present as isolated tumor-like lesions of the head and neck. The objective of the current study was to describe the cranial base manifestations of IgG4-RD. METHODS: Review of all cases at three tertiary-referral centers since disease description in 2003. RESULTS: Eleven patients were identified at a median age at presentation of 58 years (IQR, 38-65; 55% male). Ten (91%) patients had isolated skull base masses without systemic disease. Cranial neuropathies were commonly observed in the abducens (45%), trigeminal (18%), and facial nerves (18%). Lesions frequently involved the cavernous sinus (55%; 6/11) with extension to the petroclival junction in 50% (3/6). Infiltration of the internal auditory canal was present in 27% (3/11) with one case demonstrating erosion of the bony labyrinth. Preliminary clinical diagnoses commonly included nasopharyngeal cancer, pituitary macroadenoma, cholesteatoma, and meningioma / multiple meningioma syndrome. Local biopsy demonstrated >30 IgG4-positive plasma cells per high-powered field or an IgG4:IgG ratio greater than 40% in all cases. Rapid and durable clinical improvement was seen in 91% following corticosteroid and rituximab therapy. CONCLUSIONS: IgG4-RD nonspecifically presents as a rare cause of the skull base mass. Often presenting without concomitant systemic disease, local diagnostic biopsies are required. Obtaining adequate tissue specimen is complicated by densely fibrotic cranial base lesions that are frequently in close proximity to critical neurovascular structures. Primary medical therapy with corticosteroids and rituximab is effective in most patients. LEVEL OF EVIDENCE: 4 Laryngoscope, 130:2574-2580, 2020.

A Start Codon Variant in NOG Underlies Symphalangism and Ossicular Chain Malformations Affecting Both the Incus and the Stapes.

We performed exome sequencing to evaluate the underlying molecular cause of a patient with bilateral conductive hearing loss due to multiple ossicular abnormalities as well as symphalangism of the fifth digits. This leads to the identification of a novel heterozygous start codon variant in the NOG gene (c.2T>C:p.Met1?) that hinders normal translation of the noggin protein. Variants in NOG lead to a spectrum of otologic, digit, and joint abnormalities, a combination suggested to be referred to as NOG-related-symphalangism spectrum disorder (NOG-SSD). Conductive hearing loss from such variants may stem from stapes footplate ankylosis, fixation of the malleoincudal joint, or fixation of the incus short process. In this case, the constellation of both stapes and incus fixation, an exceptionally tall stapes suprastructure, thickened long process of the incus, and enlarged incus body was encountered, leading to distinct challenges during otologic surgery to improve hearing thresholds. This case highlights multiple abnormalities to the ossicular chain in a patient with a start codon variant in NOG. We provide detailed imaging data on these malformations as well as surgical considerations and outcomes.

Esophageal Perforation due to Anterior Cervical Spine Hardware Placement: Case Series.

Context. This case series discusses surgical management of esophageal perforations that occurred following cervical spine hardware placement. Purpose. (1) Determine presenting symptoms of esophageal perforation after anterior cervical spine hardware placement. (2) Discuss surgical management of these resulting esophageal perforation complications. Design/Setting. Case series of six patients at a tertiary-care, academic medical center. Patient Sample. Six patients with pharyngoesophageal perforations following anterior cervical spine surgery (ACSS). Outcome Measures. Date of ACSS, indication for ACSS, level of hardware, location of esophageal or pharyngeal injury, symptoms at presentation, surgical intervention, type of reconstruction flap, wound culture flora, and antibiotic choice. Methods. A retrospective review of patients with an esophageal or hypopharyngeal injury in the setting of prior ACSS managed by the otolaryngology service at a tertiary, academic center between January 2015 and January 2019. Results. Six patients who experienced pharyngoesophageal perforation following ACSS are included in this study. Range of presentation was two weeks to eight years following initial hardware placement. Five patients presented with an abscess and all had evidence of perforation on initial CT or esophagram. All patients underwent repair with a sternocleidomastoid flap with two patients eventually requiring an additional pectoralis myofascial flap for a persistent esophageal leak. Five patients eventually attained ability to tolerate oral nutrition. An algorithm detailing surgical reconstructive management is proposed. Conclusions. Esophageal perforations in the setting of prior ACSS are challenging clinical problems faced by otolaryngologists. Consideration should be given to early drainage of abscesses and spine surgery evaluation. Spinal hardware removal is recommended whenever possible. Utilization of a pedicled muscle flap reinforces primary closure and allows coverage of the vertebral bony defect. Nutrition, thyroid repletion, and culture-directed IV antibiotics are necessary to optimize esophageal perforation repair.

An Analysis of Risk Factors in Unilateral Versus Bilateral Hearing Loss.

A retrospective review of children with confirmed hearing loss identified through universal newborn hearing screening (UNHS) in Virginia from 2010 to 2014 was conducted in order to compare the incidence of Joint Committee on Infant Hearing (JCIH) risk factors in children with unilateral hearing loss (UHL) to bilateral hearing loss (BHL). Over the 5-year study period, 1004 children (0.20% of all births) developed a confirmed hearing loss, with 544 (51%) children having at least one JCIH risk factor. Overall, 18% of children with confirmed hearing loss initially passed UNHS. Of all children with risk factors, 226 (42%) demonstrated UHL and 318 (58%) had BHL. The most common risk factors for UHL were neonatal indicators (69%), craniofacial anomalies (30%), stigmata of HL syndromes (14%), and family history (14%). The most common risk factors in BHL were neonatal indicators (49%), family history (27%), stigmata of HL syndromes (19%), and craniofacial anomalies (16%). Children with the risk factor for positive family history were more likely to have BHL, while those with craniofacial anomalies were more likely to have UHL (P < .001). Neonatal indicators were the most commonly identified risk factor in both UHL and BHL populations. Children with UHL were significantly more likely to have craniofacial anomalies, while children with BHL were more likely to have a family history of hearing loss. Further studies assessing the etiology underlying the hearing loss and risk factor associations are warranted.

Acute post-operative airway complications following anterior cervical spine surgery and the role for cricothyrotomy.

Anterior cervical spine surgery (ACSS) is a common procedure, but not without its own risks and complications. Complications that can cause airway compromise occur infrequently, but can rapidly lead to respiratory arrest, leading to severe morbidity or death. Knowing emergent post-operative airway management including surgical airway placement is critical. We aim to review the different etiologies of post-operative airway compromise following ACSS, the predictable timeline in which they occur, and the most appropriate treatment and management for each. We place special emphasis on the timing and proper surgical technique for an emergent cricothyrotomy. Angioedema is seen the earliest as a cause of post-operative airway compromise, typically within 6-12 hours. Retropharyngeal hematomas can be seen between 6-24 hours, most commonly within 12 hours. Pharyngolaryngeal edema is seen within 24-72 hours. After 72 hours, retropharyngeal abscess is the most likely etiology. Several studies have utilized delayed extubation protocols following ACSS based on patient risk factors and found reduced postoperative airway complications and reintubation rates. The administration of perioperative corticosteroids continues to be controversial with high-level studies recommending both for and against their use. Animal studies showed that after cardiac arrest, the brain can recover if oxygenation is restored within 5 minutes, but this time is likely shorter with asphyxia prior to cardiac arrest. Experience and training are essential to reduce the time for successful cricothyrotomy placement. Physicians must be prepared to diagnose and treat acute postoperative airway complications following ACSS to prevent anoxic brain injury or death. If emergent intubation cannot be accomplished on the first attempt, physicians should not delay placement of a surgical airway such as cricothyrotomy.

Bone Conduction Implants for Hearing Rehabilitation in Skull Base Tumor Patients.

Bone conduction implants transfer sound to the inner ear through direct vibration of the skull. In patients with skull base tumors and infections, these devices can bypass a dysfunctional ear canal and/or middle ear. Though not all skull base surgery patients opt for bone conduction hearing rehabilitation, a variety of these devices have been developed and marketed over time. This article reviews the evolution and existing state of bone conduction technology.

Rhytidectomy in Patients with Cochlear Implants.

As the incidence of rhytidectomy and cochlear implantation increases, so does the likelihood that an individual patient will undergo both procedures over their lifetime. This is particularly relevant as the US population not only ages but ages well. The purpose of this paper is to describe cochlear implants and to characterize pre-, intra-, and postoperative considerations of rhytidectomy in patients with cochlear implants. A thorough understanding of the relevant anatomy and physiology is crucial to the avoidance of potentially serious complications in patients with cochlear implants.

Analysis of risk factors associated with unilateral hearing loss in children who initially passed newborn hearing screening.

OBJECTIVE: To analyze 2007 Joint Committee on Infant Hearing (JCIH) risk factors in children with confirmed unilateral hearing loss (UHL) who initially passed newborn hearing screening. METHODS: Retrospective record review of 16,108 infants who passed newborn hearing screening but had one or more JCIH risk factors prompting subsequent follow-up through the universal newborn hearing screening (UNHS) program in Virginia from 2010 to 2012. The study was reviewed and qualified as exempt by the Virginia Commonwealth University Institutional Review Board (IRB) and the Virginia Department of Health. RESULTS: Over the 2-year study period, 14896 (4.9% of total births) children passed UNHS but had the presence of one or more JCIH risk factor. Ultimately, we identified 121 babies from this group with confirmed hearing loss (0.7%), with 48 babies (0.2%) showing UHL. The most common risk factors associated with the development of confirmed UHL after passing the initial screen were neonatal indicators, craniofacial anomalies, family history, and stigmata of syndrome associated with hearing loss. CONCLUSION: Neonatal indicators and craniofacial anomalies were the categories most often found in children with confirmed unilateral hearing loss who initially passed their newborn hearing screen. While neonatal indicators were also the most common associated risk factor in all hearing loss, craniofacial abnormalities are relatively more common in children with UHL who initially passed newborn hearing screening. Further studies assessing the etiology underlying the hearing loss and risk factor associations are warranted.